West Indian Med J 2017; 66 (1): 189

189

DOI: 10.7727/wimj.2014.250

Congenital Unilateral Infiltrating Facial Lipomatosis

The Editor,

Sir,

Slavin et al in 1983 described Congenital diffuse infiltratinglipomatosis of the face (CDIL-F), which is an unfamiliar clin-ical entity characterized by collections of nonencapsulated,mature adipocytes that infiltrate locally, leading to craniofa-cial deformities with a tendency for recurrence after surgery(1). Affected children do not have abnormal psychomotor de-velopment but the facial asymmetry causes cosmetic concerns.We present the radiological findings in our case.

A one-year-old female presented with progressively in-creasing swelling over the right face since birth with the earlyeruption of deciduous teeth. The swelling on the right side ofthe face was soft, non-tender, ill-defined and diffuse causingunilateral facial hypertrophy (Fig. 1).

Congenital diffuse infiltrating lipomatosis of the face ischaracterized by nonencapsulated proliferation of mature adi-pose tissue causing diffuse infiltration of muscle and adjacentsoft-tissue. There are presence of fibrous tissue, nerve bun-dles and vessels with a thickened wall without lipoblasts andsigns of malignancy. Skeletal findings in CDIL-F are sclero-sis and the hyperplasia of the skull, cervical vertebrae, hemi-mandibular hyperplasia (of the ramus and/or condyle),accelerated dentoskeletal growth and zygomatic hyperplasia(2).

Differential diagnoses of CDIL-F are lymphatic or vas-cular malformations, lipomas (intermuscular/intramuscular,angiolipomas/fibrolipomas, liposarcoma and lipoblastomato-sis) and congenital overgrowth syndromes causing progressivehemifacial hyperplasia.

Computed tomography of the head and face showed fattyinfiltration (-25 to -100 Hounsfield units) of the right half ofthe cheek, upper and lower lips, buccinators, masseters, para-pharyngeal space and infratemporal fossa (Fig. 2).

The right parotid gland showed fatty atrophy with lipo-matous infiltration of the right half of the tongue. The righthemi-mandible was enlarged, distorted and showed fatty at-tenuation within it. Magnetic resonance imaging (MRI) con-firmed the fatty infiltration and showed the absence of areasof soft-tissue intensity (Fig.3). Biopsy through the right cheekshowed unencapsulated, lobulated, infiltration of mature fatcells without any atypical cells. Based on the clinical, radio-logical and histopathological features, a diagnosis of congen-ital diffuse infiltrating lipomatosis of the face (CDIL-F) wasmade.

Fig. 1: Computed tomographic soft-tissue rendered three-dimensional vol-umetric reconstruction. There is right facial asymmetry and largepreauricular and cheek swelling.

Fig. 2: Axial non-contrast computed tomography. There is fatty infiltrationin the right buccinator region (down arrow) with lipomatous atrophyof parotid gland (right arrow).

Fig. 3: Coronal T1 Weighted MRI. There are hyperplastic subcutaneous fatand diffuse fatty infiltration in the right buccal, masseteric, parotidand parapharyngeal spaces.

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The role of sonography, computed tomography (CT) andmagnetic resonance imaging (MRI) is very contributory inmaking the diagnosis. Ultrasound helps in identifying the dif-fuse, fatty, infiltrative and non-vascular nature of the lesion butits true anatomical extent can be depicted by cross-sectionalimaging only. Computed tomography scan shows a diffuse,non-uniform fatty infiltration with Hounsfield Unit being inthe negative range of fat along with a unilateral hyperplasia ofcraniofacial bones as seen in the present case. The predomi-nantly fatty nature of the condition is confirmed on MRI. Inchildren, the lesion exhibiting high signal intensity (isointenseto fat) on T1 and T2-weighted images and hypointensity onfat-suppressed sequences along with strong clinical correlationeliminate the need for biopsy.

This entity is managed primarily by wide local excision(3).

Keywords: Congenital, face, lipomatosis

SV KashikarFrom: Department of Radiology, Jawaharlal Nehru Medical College, Sawangi Meghe, Wardha, India.

Correspondence: Dr SV Kashikar, Behind Magnanwadi, Ramnagar, Wardha, Maharashtra 442001, India. Email: shivalikashikar@gmail.com

REFERENCES1. Slavin SA, Baker DC, McCarthy JG, Muffarij A. Congenital infiltrating

lipomatosis of the face: clinicopathologic evaluation and treatment. Plast Reconstr Surg1983; 72: 158–64.

2. Bouletreau P, Breton P, Freidel M. Congenital infiltrating lipomatosis ofthe face: case report. J Oral Maxillofac Surg 2000; 58: 807–10.

3. Padwa BL, Mulliken JB. Facial infiltrating lipomatosis. Plast Reconstr Surg 2001; 108: 1544–4.

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